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Treatment of multicentric Castleman disease

Multicentric Castleman disease (MCD) is usually much harder to treat than localized CD. There is no standard therapy for MCD, and no single treatment works for all patients. Several types of treatment have been shown to help some patients. But because MCD is rare, it has been hard for doctors to compare different treatments against each other in clinical trials.

Surgery is used to biopsy an affected lymph node to make the diagnosis, but MCD is too widespread to remove it all with surgery or treat it effectively with radiation. Still, some people are helped by these treatments. For example, surgery is sometimes used to remove an enlarged spleen that is causing symptoms, or radiation can be used to shrink tumors in a specific area that are causing problems.

More often, doctors use treatments that can reach all parts of the body, such as corticosteroids, chemotherapy, or immunotherapy, when treating MCD. Doctors usually try one or a combination of these treatments to try to put the disease into remission. Anti-viral drugs including anti-HIV treatment may also help.

In some patients the disease shrinks or even goes away completely with treatment, at least for a time. In others, the benefit does not last long and symptoms come back once treatment is stopped. Some patients may not be helped by these drugs at all.

In people whose MCD is no longer responding to other treatments, some doctors might recommend high-dose chemotherapy followed by a stem cell transplant. There are a few reported cases of this being successful. This is a complex, serious, and often expensive treatment, so it’s important to understand what it might entail if you are considering this option. For more on this treatment, see our document Stem Cell Transplant (Peripheral Blood, Bone Marrow, and Cord Blood Transplants).

The long-term outlook (prognosis) for people with MCD is often not as good as for people with localized CD. Treatment can often help for a time, but the disease tends to come back within a couple of years. A major concern is that people with MCD are at risk of dying from other causes, like serious infections or progression of the MCD to a fast-growing form of lymphoma that is hard to treat. (For more information about lymphoma, see our document Non-Hodgkin Lymphoma.)

The outlook for MCD tends to be worse if the person also has HIV/AIDS. Even if the HIV infection is under control with drug treatment, the MCD is not likely to go away. The treatment and outlook of MCD can also be complicated by the presence of Kaposi sarcoma and other AIDS-related conditions. These conditions may be less of a problem if the patient is on anti-HIV treatment.